19例珠蛋白生成障碍性贫血合并肺动脉高压患者的回顾性分析

A retrospective analysis of 19 patients with thalassemia complicated with pulmonary hypertension

目的回顾性分析珠蛋白生成障碍性贫血（TM）合并肺动脉高压（PH）患者的治疗及随访结果．探讨PH对TM的影响。方法选择2007年6月至2011年7月天津医科大学心血管病临床学院及桂林医学院附属医院收治的TM合并PH患者19例作为病例组，同时选取性别、年龄相匹配的单纯Tbl患者22例作为对照组，观察血红蛋白（Hb）、乳酸脱氢酶（LDH）、血清铁蛋白（SF）、三尖瓣返流速度（TRV）、左心室射血分数（LVEF）等临床指标，并进行生存随访4—24个月。结果病例组SF[（693．6±234．6）μg／L]、TRV[（2．6±0．1）m／s]显著高于对照组[（209．5±100．1）μg／L、（2．14-0．3）m／s，t值分别为10．79、8．92，P均〈0．05]，而两组之间Hb[（90．8±10．7）、（89．3±10．5）μg/L]、LDH[（320．9±103．7）、（355．8±140．3）U／L]及LVEF[（66．2±7．1）％、（64．2±4．7）％]比较，差异均无统计学意义（t值分别为0．44、0．50、1．05，P均〉0．05）；经生存分析Logrank检验。病例组预后较差（X2=4．95，P〈0．05）；多元线性回归分析提示，年龄、SF与TRV有关，其中sF对TRV影响较大。结论TM合并PH患者的预后较差，年龄及SF含量与PH的发展有关。

Objective To analyze the clinical feature of thalassemia with pulmonary hypertension and investigate the influence of pulmonary hypertension on thalassemia. Methods From June 2007 to July 2011, clinical data of 19 patients with thalassemia complicated with pulmonary hypertension in TEDA International Cardiovascular Hospital of Tianjin Medical University and Affilated Hospital of Guilin Medical College were analyzed retrospectively. Twenty-two cases of thalassemia without pulmonary hypertension were as a control group, and follow-up time was set as 4 months to 24 months. Observed indicators included age, hemoglobin （Hb）, lactate dehydrogenase（LDH）, serum ferritin（SF）, tricuspid regurgitation velocity（TRV） and left ventrieular ejection fraction （LVEF）. Results SF and TRV of the ease group[ （693.6 ± 234.6）μg/L and （2.6 ± 0.1）m/s] were significantly higher than those of the control[ （209.5 ± 100.1） μg/L and （2.1 ± 0.3）m/s, all P 〈 0.05]; but the differences between case and control group in Hb[ （90.8 ± 10.7）g/L vs （89.3 ± 10.5）g/L], LDH[ （320.9 ± 103.7）U/L vs （355.8 ± 140.3）U/L] and LVEF[ （66.2 ± 7.1）% vs （64.2 ± 4.7）%1 were not statistically significant （all P 〉 0.05 ）. Logrank analysis showed that the prognosis was poor in patients with thalassemia complicated with pulmonary hypertension （X2 = 4.95, P 〈 0.05）. Multiple regression analysis indicated that age and serum ferritin remained as predisposing risk factors for tricuspid regurgitation velocity, and serum ferritin had a greater impact on ihe velocity. Conclusion In patients with thalassemia complicated with pulmonary hypertension, the prognosis is poor; age and SF may be factors involved in the development of pulmonary hypertension.